Year: 2011
Type: article
Abstract: Chronic active Epstein Barr virus infection (CAEBV) encompasses a variety of EBV-associated lymphoproliferative diseases (LPD) in the non-immunocompromised host. In CAEBV either B, T, and/or NK cells are primarily infected with EBV and patients present with a variety of clinical signs and symptoms including fever, hepatosplenomegaly, and lymphadenopathy. While rare in the Western hemisphere, T and NK cell CAEBV is most commonly seen in Japan. In Japan, current treatment protocols rely on chemotherapy and hematopoietic stem cell transplantation (HSCT). To evaluate the clinical outcome of HSCT in CAEBV patients in the United States, we reviewed our experience with 6 CAEBV patients (4 T-cell, 1 NK-cell, 1 B-cell), who underwent allogeneic HSCT for their disease. Median age at transplant was 11 yrs (range 6 – 25 yrs) and the median time to transplant from diagnosis was 3.7 years (range 4.3 yrs – 8.5 yrs). Four of 6 patients had persistent or refractory disease at the time of HSCT. Four patients received myeloablative conditioning while 2 patients received reduced intensity conditioning (RIC). Median time to neutrophil engraftment was 16 days (range days 11 – 21 days) with no long-term engraftment failure. Five patients received infusions of donor derived EBV-specific cytotoxic T cells (CTLs) at a median of 6 months (range 2.5 months – 12 months) post HSCT. Five patients are alive and in complete remission with a median survival of 2.5 yrs (range 7 months – 10 yrs). One patient developed recurrent T-cell CAEBV immediately post transplant and died of an aggressive T-cell lymphoma 3.7 years post transplant. Our results support published data from Japan showing good outcomes for CAEBV patients after allogeneic HSCT. Factors that may influence outcome and warrant further study include the timing of transplantation after diagnosis and the use of EBV-directed T-cell therapies.
Authors: J.A. Craddock, C.M. Bollard, R.A. Krance, M.J. Cowan, M.S. Cairo, H.E. Heslop, S. Gottschalk
Language: English
Subfield: Oncology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $3,650
OpenAlex ID: W2034959244
